English - International Waldenstrom's Macroglobulinemia Foundation

Letter to the Editor
Each issue of the Torch invites readers to “Have your say” by sending letters, articles, or suggestions for articles to
the editor of the newsletter. Recently the Torch received a letter from Raphael Altman, an IWMF member in England,
outlining what he perceived to be disinterest in IWMF publications and in the medical community at large concerning
the rare occurrence of tumors in WM. This letter is printed in full below. The response which follows was written by Dr.
Robert A. Kyle, IWMF Trustee and Chairman of the IWMF Scientific Advisory Committee.
Alice Riginos
Torch Editor
Dear Editor,
I am addressing the topic: WM, Tumors, and the IWMF.
I have surveyed IWMF-Talk and found that of 1,000
members, 10 have solid tumors. That’s 1%. Eight of these
ten have enough abnormal IgM to be diagnosed WM but
not enough to be a problem. Steven Treon states, “Most
cases of LPL are WM, with less than 5% of cases made up
of IgA, IgG and non-secreting LPL.” (All WM patients
have underlying LPL) People reporting on IWMF-Talk
have had tumors on the spine, in the foot, in the mouth –
it seems like they can appear anywhere.
A word about words. Many doctors appear to be satisfied
with the term ‘solid tumor’ to describe this phenomenon.
Others suggest alternative terms such as ‘mass’ or even
‘aggregations of lymph tissue cells.’ What we decide to
call the condition is less important than the implications
of having it.
I have observed that there is currently no mention of
it on the IWMF website. Nor is it mentioned in the
‘Introduction to WM’ or ‘For the newly diagnosed’ or
‘When to treat’ type of articles that have appeared in
the Torch or in similarly-titled talks given at the annual
Educational Forum. I believe this is an omission that
stands to be corrected.
Firstly, the simple acknowledgement of a form of the
disease affecting probably 1+% of patients would seem
to be reasonable. Secondly, particular observations – and
even suggested treatments? – relating to this section of
patients might then have a greater chance of appearing
on the WM radar. Thirdly, patients who might only
monitor their IgM as a disease indicator would be less
likely to make the mistake of thinking that good IgM
readings alone are necessarily an indicator that all is well,
when they could have indolent tumors quietly growing
undetected.
For example: my first tumour (6 cm), inside the spinal
column, was spotted because at diagnosis I presented
with some back pain and was scanned. My second tumour
(12 cm), on the outside of the spine, was missed, possibly
for as long as 2 years, because my IgM counts continued
to be fine and dandy. (It was eventually spotted because
I started to experience a similar, albeit mild, back pain)
Risks of extra exposure to radiation notwithstanding, the
dominant view among those on IWMF-Talk with tumors
appears to be to scan at diagnosis and/or whenever
unexplained pains might warrant scanning. Once
someone has had a tumor, occasional scanning to pick up
anything untoward could be considered.
As to the science of what is going on with these patients
(let alone why this happens to particular individuals) there
appears to be not a great deal available on the subject,
presumably (and understandably) because researchers
and clinicians are concentrating on the issues of elevated
IgM which afflict the vast majority of patients.
Accepting the caveat that there are areas where the clear
distinction between B-cells and plasma cells becomes
less clear and where it even becomes unclear as to which
cells are proliferative and which are IgM secreting,
nevertheless in his paper ‘Pathological findings in WM’
Dr. Roger Owen writes “The dominant component is
comprised of B-cells which are considered to be the
proliferative fraction … the second cellular component
comprises plasma cells which are responsible for the
production of IgM.” Elsewhere he states: “The clinical
features of WM will likely form a continuous spectrum
with the high IgM / hyperviscosity patients at one end
and the patients with bulky lymph node disease (and
tumors? – RA) and low IgM at the other.”
Raphael Altman
England
RESPONSE
Mr. Altman has raised an important point about
Waldenstrom’s macroglobulinemia. When one mentions
“solid tumors,” the hematologist/oncologist thinks first of
cancer of the lung, colon, prostate, breast, etc., while Mr.
Altman is referring to a mass which consists of malignant
lymphocytes and plasma cells that are identical to those
seen in the bone marrow of patients with WM. The bone
marrow, lymph nodes and spleen are the usual sites of
WM, but in a small number of patients (approximately
5%), masses or tumors of the malignant lymphocytes and
plasma cells are found outside the bone marrow and the
lymph nodes.
Letter to the Editor, cont. on page 13
12 IWMF TORCH Volume 11.2